Mouth Diseases

Jaw Cancer

04/28/2000

Question:

A family member had an abcessed tooth removed and upon the subsequent analysis of the pathology report was diagnosed with Jaw Cancer, specifically Savour Cystic Ameloblastic Carcinoma with predeterminate squamous at differentiation....an apparently very rare form of cancer....we are trying in vain to locate information on this cancer, any info you could provide would be helpful

Answer:

Some of the terms you have used are familiar, while others (particularly Savour?) are not. Hopefully, the following will apply to your family member.

Ameloblastic Carcinoma (or Cystic Ameloblastic Carcinoma) is an extremely rare form of cancer that arises from cells within the jaws that were part of the process involved in the formation of teeth. In most cases, after the teeth erupt, these cells remain quiet and do not proliferate. Sometimes, as with an abscessed tooth, the cells may be stimulated to form a cyst. A cyst is one way in which the body localizes infection or degenerative material. Less commonly, these cells can spontaneously start to grow, leading to a variety of benign tumors. The ameloblastoma is an example of such a tumor. During tooth development, the ameloblast is the specific cell that produces the hard enamel that forms the outer surface of our teeth.

Although considered benign, the ameloblastoma has a troublesome pattern of growth, termed infiltrative. This means that small strands or cystic bubbles of the tumor sneak into the surrounding tissues (particularly bone) on an individual basis. Such growth is impossible to see with the naked eye. Therefore, even with apparently adequate surgical removal, ameloblastoma has the tendency to recur or regrow from microscopic bits of tumor that were inadvertently left behind.

Ameloblastic Carcinoma is the malignant counterpart of ameloblastoma. Malignant is the term used to describe tumors that can metastasize (travel to lymph nodes or distant sites, like the lung). Unfortunately, because they are extremely rare (less than 60 cases have been reported in the entire English literature), it is nearly impossible to accurately predict the behavior of an individual tumor. As with most other malignancies, if the cancer is found only locally, the patients tend to do better following surgical excision. In cases where metastatic tumor deposits are found, the reported long-term survival is about 50%.

Finally, since the diagnosis is so uncommon, it is probably worthwhile for the family member to request a second opinion on the biopsy material. I would recommend that the slides be reviewed by an oral and maxillofacial (OMF) pathologist. These specialists are dentists who have received additional training in the diagnosis and treatment of tumors and diseases of the jaws and mouth. They are usually found at universities that have a dental school. Since they specialize in the oral and facial area, OMF pathologists typically have far greater experience with tooth-related specimens than general pathologists. Even if the original diagnosis was made by an experienced OMF pathologist, a second opinion to confirm this unusual diagnosis would be most reasonable.

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